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May 2020 -
Volume 18, Issue 5

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From the Editor

Editorial
A. Abyad (Chief Editor)
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Letter to the Editor
Impact of Age Factor in COVID-19 Infectivity in Population of Nowshera KP, Pakistan
Hamzullah Khan
DOI: 10.5742MEWFM.2020.93813

Original Contribution

[Abstract]
Physician’s Satisfaction Regarding Plan of Care among Physicians in Abha City, Saudi Arabia, 2018
Maher Abdulkarim Alsmail, Safar A. Alsaleem, Bandar Alasmari
[pdf]
DOI: 10.5742MEWFM.2020.93804


[Abstract]
Adult Allergic Rhinitis in Aseer, Southwestern Region of Saudi Arabia: Prevalence and its Concomitant Aspects
Badr Al-Ghamdi
[pdf]
DOI: 10.5742MEWFM.2020.93805

Population and Community Health

[Abstract]
Knowledge and attitude of dyslipidemia among school teachers in Ahad Rufaidah, Aseer Region, Saudi Arabia
Mansoor Abdullah Al-Lajhar, Mohammad S. Al-Shahrani, Naif M. Alqahtani
[pdf]
DOI: 10.5742MEWFM.2020.93806

[Abstract]
The safest values of high density lipoproteins in the plasma
Mehmet Rami Helvaci, Abdulrazak Abyad, Lesley Pocock
[pdf]
DOI: 10.5742MEWFM.2020.93807

Clinical Research and Methods

[Abstract]
Intra-articular injection of hyaluronic acid in patients with knee osteoarthritis in Aden, Yemen
Abdulsalam Abdullah Hadi Mohsen
[pdf]
DOI: 10.5742MEWFM.2020.93808

Education and Training

[Abstract]
Minor Surgery in Primary Care; Audit Report
Muhammad Hameed, Shabana Shaheen
[pdf]
DOI: 10.5742MEWFM.2020.93809

Reviews

[Abstract]
Food allergies in atopic dermatitis
Idris Akinwande, Kazeem Salako
[pdf]
DOI: 10.5742MEWFM.2020.93810

[Abstract]
Parkinson’s Disease: An update on Pathophysiology, Epidemiology, Diagnosis and Management.
Part 1 : Background and Epidemiology
Abdulrazak Abyad
[pdf]
DOI: 10.5742MEWFM.2020.93811

Case Report

[Abstract]
Congenital Acinar Dysplasia: A Familial Cause of Severe Primary Lung Hypoplasia
Ashwag Asiri, Ali Alsuheel Asseri , Ahmed Aldurah, Eman Saeed Sallem , Bayan Hanif Al-Qahtani
[pdf]
DOI: 10.5742MEWFM.2020.93812


Middle East Quality Improvement Program
(MEQUIP QI&CPD)

Chief Editor -
Abdulrazak Abyad MD, MPH, MBA, AGSF, AFCHSE
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AUSTRALIA
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May 2020 - Volume 18, Issue 5

Congenital Acinar Dysplasia: A Familial Cause of Severe Primary Lung Hypoplasia


Ashwag Asiri (1)
Ali Alsuheel Asseri (1)
Ahmed Aldurah (2)
Eman Saeed Sallem (3)
Bayan Hanif Al-Qahtani (3)

(1) Department of Child Health, King Khalid University, Abha, Saudi Arabia
(2) Neonatology Consultant, Maternity and Children Hospital, Khamis Mushayt, Saudi Arabia
(3) Final year MBBS student, King Khalid University, Abha, Saudi Arabia

Corresponding author:
Ashwag Asiri
Department of Child Health, King Khalid University, Abha, Saudi Arabia
Phone number: +966542957779
Email: jory_1411@hotmail.com

Received: March 2020; Accepted: April 2020; Published: May 1, 2020. Citation: Ashwag Asiri et al. Congenital Acinar Dysplasia: A Familial Cause of Severe Primary Lung Hypoplasia. World Family Medicine. 2020; 18(5): 72-74 DOI: 10.5742MEWFM.2020.93812

Abstract

Objectives: To report a Saudi family of consanguineous parents who had two daughters with familial congenital acinar dysplasia, who died shortly after birth of respiratory failure.

Case Presentation: A full-term female baby born to a 28-year-old mother via emergency Cesarean section. Antenatally, the mother was diagnosed with preeclampsia and severe oligohydramnios. The baby developed severe respiratory distress immediately after birth and required positive pressure ventilation in the operating room. Echocardiography revealed severe pulmonary hypertension with supra-systemic estimated pulmonary pressure. Despite all management measures, the baby continued to deteriorate with persistent respiratory failure. The diagnosis of CAD was confirmed by open lung biopsy at the age of two months. She passed away at the age of three months due to severe refractory respiratory failure. One year later, her mother delivered another baby girl with CAD who also died of respiratory failure at the age of two months.

Conclusions: CAD is a rare cause of lung hypoplasia. It mainly affects females and its etiology may be through autosomal recessive inheritance. The affected child usually dies of respiratory failure shortly after birth. It should be expected prenatally if there is absence of fetal breathing movements. Fetal monitoring and proper antenatal care may have a role in prevention of CAD.

Key words: Congenital acinar dysplasia, Consanguinity, Primary lung hypoplasia,
Respiratory insufficiency.

 







 


 
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